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Congenital

Genitourinary
Abnormalities

By: Kumala

Genitourinary Tract
Development

A number of developmental abnormalities can lead to infertility,


subfertility, spontaneous abortion, or midpregnancy and
preterm delivery.

Embryologically in females, the external genitalia, gonads, and


mllerian ducts each derive from different primordia and in
close association with the urinary tract and hindgut.
Abnormal embryogenesis of these is thought to be multifactorial

Embryology of the urinary system

Beetwen the 3rd and 5th gestasional weeks, an elevation of


intermediate mesoderm on each side of the fetus-the urogenital
ridge-begin develops into the urogenital tract, which will
become the ovary.
The Mullerian ducts become the fallopian tubes, uterus and
vagina.
The urinary tract develops from the mesenephros or wolfian
ducts connect the mesonephric kidney to the cloaca (Fig.3-1A)

Beetwen the 4th-5th weeks, each mesonephric ducts give


rise to uteric bud (Fig.3-1b)
As each bud lenghtens, it induces differentiation of the
metanephros, which will become the final kidney (Fig.3-1C)
The cloaca begins as a common opening for the embryonic
urinary, genital, and alimentary tracts.

At 7th week the cloaca becomes divided by the urorectal


septum to create the rectum and urogenital sinus (Fig.3-1D)
The urogenital sinus is considered in three parts: (1) the
cephalad or vesicle portion,which will form the urinary bladder;
(2) the middle or pelvic portion, which creates the female
urethra; and (3) the caudal which will give rise to the distal
vagina and to the greater vestibular (Bartholin) and
paraurethral (Skene) glands

Embryology of the genital tract

Development of the genital tract begins as the


mullerian ducts, also termed paramesonephric
ducts, from the lateral to each mesonephros.
The uterus is formed by this union of the two
mullerian ducts at approximately the 10 th week
(Fig.3-1E)
Fusion begins in the middle and then extends
caudally and chepalad (Fig.3-F)

the final uterine cavity is usually shaped by the 20th week.

If the two mllerian ducts fail to fuse, then there are separate
uterine horns.

The close association of the mesonephric (wolffian) and


paramesonephric (mllerian) ducts explains why there are
commonly simultaneous abnormalities involving these
structures.

Anomalies most frequently associated with renal defects are


unicornuate uterus, uterine didelphys, and agenesis syndromes,
whereas arcuate and bicornuate are less commonly linked
(Reichman, 2010).

Mesonephric Remnant
Mesonepric or wolffian vestiges can
persist as gartner duct cysts. These
are typically located in the proximal
anterolateral vagina wall.
Intraabdominal wolffian remnant
include a few blind tubules in
mesivarium

The epophoron or parophoron may develop into clinically


identifiable cysts and are included in the differential
diagnosis of an adnexal mass

Bladder and Perineal Abnormalities

Very early during embryo formation , a bilaminar cloacal


membranes lies at the caudal end of the germinal disc and
forms the infraumbilical abdominal wall.
Normally, an ingrowth of mesoderm between the
ectodermal and endodermal.
Without reinforcement, the cloacal membrane may
prematurely rupture, and depending on the extend of the
infraumbilical defect, cloacal exstrophy, bladder exstrophy,
or epispadias

Defects of the hymen

Hymeneal anomalies include:


Imperforate
Microperforate
Cribiform
Navicular and
Septate hymens

Mullerian Abnormalities
There are four principal deformities of mullerian ducts:
1.

agenesis of both ducts

unilateral maturation of one mllerian duct with incomplete

Absent midline fusion of the ducts

defective canalization

Vaginal Abnormalities
There are two types of congenital septa:
1.

Longitudinal septum: which arises from a fusion or


resorption defect.

2.

Transverse septum: result either from incomplete


canalization

Cervical Abnormalities

1. Partial or complete agenesis


2. Duplication
3. Longitudinal septa

Uterine Abnormalities

Unicornuate

the underdeveloped or rudimentary horn


may be absent.
General population estimeted in 1 in 4000
women.
It may be detected during fertility
evaluation by HSG

Uterine didelphys (class iii)

This anomaly arises from a complete lack of fusion that result in


two entirely separate hemiuteri, cervices and usually two
vaginas
Most women have a duoble vagina or a longitudinal vaginal
septum

Uterine didelphys may be isolated or part of a triad that has an


obstructed hemivagina and ipsilateral renal agenesis

Bicornuate uterus (class iv)


This relatively common anomaly
forms as lack of fundal fusion
results in two hemiuteri, with only
one cervix and vagina
a coexistent longitudinal vaginal
septum is not uncommon.

Septate uterus (class v)

This anomaly is caused when a resorption defect leads to a


persistent complete or partial longitudinal uterine cavity septum
Septate anomalies are associated with diminished fertility as
well as increased risks for adverse pregnancy outcomes that
include miscarriage, preterm delivery, and malpresentation

Arcuate uterus (class vi)


This malformation is a mild
deviation from the normally
developed uterus

Diethylstilbesterol Reproductive
Tract Abnormalities (Class VIII)
- During the 1960s, a synthetic nonsteroidal
estrogen diethylstilbestrol (DES)was used
to treat pregnant women for threatened
abortion, preterm labor, preeclampsia, and
diabetes.
- The treatment was remarkably ineffective.
- Increased risks of developing a number of
specific reproductive-tract anomalies
- Fertility issues

vaginal clear cell adenocarcinoma, cervical intraepithelial


neoplasia, small-cell cervical carcinoma, and vaginal adenosis

identifiable structural variations in the cervix and vagina to


include transverse septa, circumferential ridges, and cervical
collars

smaller uterine cavities, shortened upper uterine segments, Tshaped and other irregular cavities, and fallopian tube
abnormalities

Impaired conception rates and higher rates of miscarriage,


ectopic pregnancy, and preterm delivery, especially in those
with structural abnormalities

- >

50

years

after

DES

use

was

proscribed
- most

affected

women

are

past

childbearing age
- higher rates of earlier menopause and
breast cancer have been reported in
exposed women

Fallopian Tube Abnormalities


- The fallopian tubes develop from the
unpaired distal ends of the mllerian
ducts
- Congenital
anomalies
include
accessory
ostia,
complete
or
segmental tubal agenesis, and several
embryonic cystic remnants
- The most common is a small, benign
cyst attached by a pedicle to the
distal end of the fallopian tubethe
hydatid of Morgagni

UTERINE FLEXION
Anteflexion
Retroflexion
Sacculation
Uterine Torsion

Anteflexion

Defined by anterior flexion of the uterine fundus relative to the


cervix in the sagittal plane.
Mild or moderate flexion is typically without clinical
consequence.
Congenital or acquired extremes may lead to pregnancy
complications.

Retroflexion
Posterior uterine fundal flexion in the sagittal plane.
A growing retroflexed uterus will occasionally become
incarcerated in the hollow of the sacrum.
Symptoms include abdominal discomfort, pelvic pressure,
and voiding dysfunction that may include urinary
frequency or retention.

The incarcerated uterus must be


repositioned to its normal anatomical
position. After bladder catheterization,
the uterus can usually be pushed out
of the pelvis when the woman is
placed in the knee-chest position.
laparoscopy was used at 14 weeks to
reposition the uterus using the round
ligaments for traction.

Sacculation
Persistent entrapment of the pregnant uterus in the pelvis may lead to
extensive lower uterine segment dilatation to accommodate the fetus.
An elongated vagina passing above the level of a fetal head that is deeply
placed into the pelvis suggests a sacculation or an abdominal pregnancy.
Cesarean delivery is necessary when there is marked sacculation.

Sacculation

Uterine Torsion

It is common during pregnancy for the uterus to rotate to the right


side. Uterine rotation exceeds 180 degrees to cause torsion.
Most cases of torsion result from uterine leiomyomas, mllerian
anomalies, fetal malpresentation, pelvic adhesions, and laxity of the
abdominal wall or uterine ligaments.
Symptoms : obstructed labor, intestinal or urinary complaints,
abdominal pain, uterine hypertonus, vaginal bleeding, and
hypotension.
Most cases of uterine torsion are found at the time of cesarean
delivery

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